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Type: Journal article
Title: Paranodal pathology in Tangier disease with remitting-relapsing multifocal neuropathy
Author: Cai, Z.
Blumbergs, P.
Cash, K.
Rice, P.
Manavis, J.
Swift, J.
Ghabriel, M.
Thompson, P.
Citation: Journal of Clinical Neuroscience, 2006; 13(4):492-497
Publisher: Churchill Livingstone
Issue Date: 2006
ISSN: 0967-5868
Statement of
Z. Cai, P.C. Blumbergs, K. Cash, P.J. Rice, J. Manavis, J. Swift, Mounir N. Ghabriel and P.D. Thompson
Abstract: Pathological studies of a sural nerve biopsy in a man with Tangier disease presenting as a remitting-relapsing multifocal neuropathy showed abnormalities in the paranodal regions, including lipid deposition (65%) and redundant myelin foldings, with various degrees of myelin splitting and vesiculation (43%) forming small tomacula and abnormal myelin terminal loops (4%). The internodal regions were normal in the majority of myelinated fibres. Abnormal lipid storage was also present in the Schwann cells of the majority of unmyelinated fibres (67%). The evidence suggests that the noncompacted myelin region of the paranode is a preferential site for lipid storage in the myelinated Schwann cell, and that the space-occupying effects of the cholesterol esters leads to paranodal malfunction and tomacula formation as the pathological basis for the multifocal relapsing-remitting clinical course.
Keywords: Tangier disease; Remitting-relapsing neuropathy; Paranode
Description: Copyright © 2006 Elsevier Ltd All rights reserved.
RMID: 0020060594
DOI: 10.1016/j.jocn.2005.07.009
Appears in Collections:Medicine publications

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